Transcript Sickle Cell Disease Erika Heard, PGY5 Internal Medicine/Psychiatry

SICKLE CELL DISEASE: OTHER ASPECTS
TO CONSIDER
ERIKA HEARD, PGY5
INTERNAL MEDICINE & PSYCHIATRY
EMORY UNIVERSITY
OCTOBER 30, 2015
Objectives:
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Inspect the impact of psychological distress on the
functioning of patients with sickle cell disease (SCD)
Identify the common the psychiatric co-morbidities in
SCD
Demonstrate an understanding of the role of
inflammation in SCD
Appreciate the effects of physician attitudes on
clinician-patient interactions
Overview: Sickle Cell Disease (SCD)
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Background
Role of inflammation
Mental Health Co-morbidities
Utility of Cognitive Behavioral Therapy
Substance Use
Physician Attitudes
Background:
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SCD is a common autosomal recessive genetic disorder 1
1/375 African Americans are affected by SCD 1
Caused by a mutation on the 6th codon of theβ globin gene
leading to a valine substituting for glutamic acid 2
Causes altered form of hemoglobin: HbSS, HbSC, HbS-β
thalssemia 1
Primarily in those of African decent but also in
Mediterranean, Middle Eastern, and Asian decent 1
Average life span of sickle red blood cell is 12-15 days
while normally it is 120 days 3
Complications of SCD leads to multiple hospitalizations with
>$450 million dollars in healthcare expenses each year 4
Inflammation in SCD:
SCD is considered a chronic inflammatory condition 2
 Increased levels of inflammatory markers due to
repeated vaso-occlusive episodes and endothelial cell
activation
 Bandeira et al, conducted a cross-sectional study of 62
patients with HbSS looking at the level of inflammatory
markers in their blood 2
- levels of IL-6 and TNF-α were significantly more
elevated and IL-7 was elevated when comparing the
study and control groups
 Consistent with past studies, even when not having a
vaso-occlusive crisis (in steady state), there is
inflammation 2
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Inflammation in SCD:
 Changes in the red blood cell
causes the cells to have a greater
affinity for the vessel walls
 This activates the endothelial cells
of the cell wall
 When the endothelial cells become
activated they release cytokines
 Monocytes undergo extravasation
 More inflammatory cells are
released and surround the nociceptor
terminals
 These terminals release substance P
 Feeling of pain is detected 4
Lutz, B., Meiler, S., Bekker, A., et al. Updated mechanisms of sickle cell diseaseassociated chronic pain. Transl Perioper Pain Med. 2015; 2(2):8-17.
Complications of SCD:
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Repeated microvascular insults lead to organ damage
affecting most systems: 1
- CVA
- Acute chest syndrome
- Avascular necrosis
- Sickle cell retinopathy
- Splenic sequestration
- Vaso-occulsive crisis
- Chronic pain
- Nephropathy
- Increased risk of infection
Co-morbid Mental Illness in SCD:
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PiSCES study looked at the health related quality of life
(HRQOL) of those with SCD compared to those who suffer
from cystic fibrosis, asthma, and on hemodialysis 5
-SCD had similar HRQOL to those with other chronic
diseases even compared to chronically debilitating illnesses
such as being on hemodialysis
The negative impact on the quality of life of those with SCD
is likely related to the chronicity of illness, chronic pain,
frequent hospitalizations, and the unpredictable nature of
the illness 5
Often symptoms such as fatigue, poor sleep, depressed
mood, etc are due to pain and these symptoms overlap with
symptoms of depression 5
Co-morbid Mental Illness in SCD:
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Rates of depression in this population vary between 18-44% 6
In the PiSCES study, the rate of depression was 27.6% (13.8% met criteria
for MDD) and 6.5% had anxiety 5
- In this patient population, patients with depression were significantly older,
poor, and suffered from more frequent days of pain than those who were
not depressed
- Depressed patients had significantly worse functioning on all short form
health survey (SF-36) subscales than those who were non-depressed
Depressed patients used more opioids and received less relief from these
medications 6
Some studies found depressed and anxious patients had greater healthcare
utilization while others did not 6
In addition to social factors such as social isolation, decreased physical
functioning, and financial hardship, there are also biological factors that
contribute to depression 6
Inflammation and Depression:
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Increasing amount of research exists that connects
psychological stress to increased inflammation leading to
behavioral changes 7
- These changes have been described as “sickness
behaviors”
Studies show that chronic inflammation may play a role in
diseases such as asthma, rheumatoid arthritis, coronary
artery disease, diabetes, inflammatory bowel disease, and
Alzheimer's 7
In these studies, there are increased rates of depression 7
- A study on rheumatoid arthritis and inflammatory bowel
disease rates of depression were 2-3 times greater than in
the general population
Inflammation and Depression:
Miller, A., Maletic, V., and Raison, C. Inflammation and its discontents: the role of cytokines in the pathophysiology of major
depression. Biol Psychiatry. 2009; 65(9): 732-714.
Inflammation and Depression:
Singhal, G., Jaehne, E., Corrigan, F., et al. Inflammasomes in neuroinflammation and changes in brain function: a focused review. Front Neurosci.
2014; 8(315): 1-13.
Inflammation and Depression:
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Weinstein et al, showed that depressed patients’
plasma levels of pro-inflammatory cytokines TNFalpha, IL-6, and CRP are elevated 7
Another study, Dowlati et al, found that these proinflammatory markers were elevated in the brains of
patients with MDD leading to neuroinflammation 7
Inflammation and depression in SCD:
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No studies found looking at how chronic
inflammation from SCD may affect mood
- However, many previous studies regarding chronic
inflammatory diseases show that this inflammation
can modulate mood and likely has an impact on
depressive symptomatology
Future studies may focus on specifically whether the
chronic inflammation in SCD correlates with
depression
Neurocognitive Effects of SCD:
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Many SCD patients are affected cognitively by
chronic anemia, pain events, and frequent use of
opioid medications 10
In addition to these factors, 10-15% of patients
with HbSS have overt strokes and 20-25% have
silent strokes 10
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Thus, many patients with SCD have unrecognized
neurocognitive issues
Cognitive impairment influences one’s ability to
transition to adulthood due to compromised executive
functioning affecting social abilities and problem
solving skills
Psychological Distress:
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SCD is a chronic disease beginning from birth
Dealing with frequent, disruptive pain episodes greatly impacts
the growth and development of children with SCD leading to 11:
- Social isolation
- Difficulty developing fulfilling peer relationships
- Academic difficulties
- Difficulty developing a cohesive identity
These difficulties in childhood can carry over to adulthood 11
- Limited studies comparing psychologically and socially
sturdiness in those with SCD compared to those without SCD
Edwards et al, found that resiliency is a factor that leads to the
difference in those with SCD who are less affected vs those who
are more affected 11
Psychological Distress:
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Many patients with SCD have significant psychological
distress 12
Maladaptive behaviors develop from this psychological
distress 12
Most patients with SCD are dealing with emotions of
anger, frustration, and fear 12
Previous studies have shown that negative mood and
stress are related to pain, psychosocial, and functional
outcomes 12
Similarly, Edward et al, demonstrated a relationship
between negative emotional reactions to pain and
psychological outcomes in SCD 11
Cognitive Behavioral Therapy (CBT):
 Goal is to help those
recognize the relationship
between thoughts, emotions,
and actions
 Understanding these
relationships and catching
automatic thoughts helps to
change one’s emotions and
one’s reactions to situations
13
https://growwiseyoga.wordpress.com/
CBT in SCD:
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With SCD, the goal is to recognize one’s thoughts
regarding his/her pain and how this influences
actions
Improve coping skills
Reduce psychological distress
Reduce fear avoidance to allow one to become
more active in life
Recognize and correct maladaptive behaviors 14
CBT in SCD:
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When treating patients with SCD, little focus has been
placed on the psychological and social cultural aspects of
pain
Studies have shown the efficacy and cost effectiveness of
cognitive behavioral interventions in addressing psychosocial
aspects of chronic pain (ie reduction in hospital admission,
analgesics intake, and decreased ER visits)
Cognitive based therapies can provide alternative coping
mechanisms leading to greater empowerment and foster
greater self-reliant 14
Grady CBT for SCD Group:
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Occurs on Wednesday afternoons in International
Clinic
Referrals accepted by Dr. Clearo by Epic messaging
her
Accepting all patients with SCD, especially high
utilizers
Will receive initial psychiatric assessment and
determine whether interested and appropriate for
the group
A Patient Perspective:
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“More times than not, when I am finally seen by a
physician or nurse, I am met with a callous, rude,
disdainful attitude…{Why are there no physicians
willing to accept new adult sickle cell patients?} Some
of the reasons that I have been given are that sickle
cell patients tend to be narcotics seekers and addicts,
that most are on welfare, that they take up an
inordinate amount of doctors’ time, and that they
tend to be difficult patients…” –Glinda DamesFincher 15
Transitions In Care:
 Previously not a chronic illness
 Transitioning from pediatric care to adult care can be
difficult
 Limitations in care transitions 16:
- Limited adult providers
- Poor communication between pediatric and adult
care centers
- Family and patient unpreparedness for transition
- Limited financial independence
Transitions In Care:
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Telfair et al, found that adolescents, young adults, and
primary caregivers endorse concerns about this transition 17
Primary concerns included:
- How they would pay for medical care
- Providers being able to understand who sickle cell affects
a person with this disease
- Staff not believing their pain
- Leaving the pediatric center
- Primary Caretaker endorsed concern that child would be
able to handle responsibility
Substance Use in SCD:
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Why do you think there is this perception of opioid addiction
in this population?
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What is the rate of opioid addiction in patients with SCD?
- Actual rates are 3-10% which is no higher than the
general population 18
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Multiple studies have shown that healthcare professionals overestimate
rates of opioid abuse by patients with sickle cell disease 19
- One study found that ½ of ED doctors and ¼ of hematologist believe
that ~20% of sickle cell patients they encounter have a problem with
opioid abuse
- Another study found that 63% of nurses believed that opioid
addiction frequently develops in sickle cell patients
Pseudoaddiction:
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Common for those on long term opioids to develop
tolerance and physiological dependence 19
Due to the development of tolerance and
dependence, MD’s can be uncomfortable to dose
these medications 18
Does anyone know about psudoaddiction?
- Manifestation of certain behaviors (exaggerated
or manipulative) based on under treatment of
pain 19
Pseudoaddiction vs Dependence:
Examples of Patients’ Descriptions of DSM-IV Symptoms of Substance Dependence
Classified as Pain-Related and Non-Pain Related 19
Pain-Related Symptoms
Non-Pain-Related Symptoms
“I found I needed more and more medication [to
manage pain at home and avoid going into
hospital]” (Tolerance)
“I couldn’t sleep without them [pain killers] I was restless at
night walking up and down and feeling depressed”
(Withdrawal symptoms)
“…Sometimes I am tempted to take it more
frequently than prescribed, say when I am still in
pain after two hours…” (Greater use than intended)
“I have taken an excess amount, because of my psychological
state; I was stressed and thinking of killing myself” (Greater
than intended use)
“I have done that before [taking more painkillers]
thinking the more I take the faster the pain would
go” (Greater use than intended)
“…I look two, three, or even four, I was like high…” (Greater
use than intended)
“…It makes my skin itch and makes me feel
drowsy…but nothing else works. I try not to take it
again but the pain always comes back” (Attempt to
cut down)
“…If the pain is really severe and I take a high dose.
The tablets make me sleepy and I have to go to bed,
so I am not able to go out” (Social impairment)
“I knew I was becoming dependent and I weaned myself off…”
(Attempted to cut down)
“I was going through problems—depression I was staying
indoors and not working and being awake all night” (Social
impairment)
A Common Grady patient:
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Ms. H is a 27 y/o AAF with pmh sig for HbSS with complications
of acute chest, stroke, and multiple hospitalizations for vasoocclusive crisis (7 in the past year) presents for pain typical of
her sickle cell pain. At home she is on MS contin 60mg BID and
10mg IR q4H. She has tried to alleviate the pain at home but
has returned to your service for the second time this month. You
remember her always talking on the phone, laughing, watching
movies, doing things that you feel was atypical for someone
“who claims” they have severe pain. You dread going down to
the ER having to face her because you know she requires dilaudid
4mg IV q4 hours and you feel uncomfortable giving her this
dose. Your resident snickers that you have the unfortunate luck to
take care of the “sickler” again. You just hope to get her out as
soon as possible so you don’t have to deal with her constant
request. She really only wants the narcotics anyway.
Physician Attitudes:
 Reflecting on the previous patient, when you have
had a patient like this admitted, what is usually your first
thought?
 How do you think these thoughts negatively or
positively affect your interaction with patients with SCD?
 What is the culture that our program promotes
when caring for SCD patients?
 What would be helpful for you as a resident to better
care for patients with SCD?
Physician Perceptions Toward SCD:
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Health disparities are prevalent 20
Factors that may influence these disparities 20:
- Provider bias/prejudice
- Clinical uncertainty when interacting with minority
populations
- Stereotypes about minority populations held by
providers
Using the term “sickler” dehumanizes an individual and
devalues them to just their illness 21
- Glassberg et al, found that there was a correlation
between negative attitudes towards patients with SCD
and the use of the term “sickler” by ED phyicians
Physician Perceptions toward SCD:
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Patients recognize these negative beliefs held
toward them and feel stigmatized 22
Effects of physician perceptions 22
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Negative view toward healthcare system
Feeds pseudoaddiction
Breakdown of the therapeutic alliance
Patient feels devalued
Poor pain control
Improving Care of Patients with SCD:
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Platt et al, based at the GA Comprehensive Sickle Cell
Center published guidelines regarding patient care
during a vaso-occlusive episode 3
A- Assessment of the pain (use a pain assessment tool)
B- Believe the patient’s level of pain
C- Complications or cause of pain
D- Drugs and distractions (opioids and NSAIDS/distract
with music, TV, relaxation techniques)
E- Environment (quite area for rest)
F- Fluids
Improving Care of Patients with SCD:
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In future encounters with SCD patients, remember
that there are social, psychological, cultural factors
that influence the current interaction between you
and patient
Gaining better context of all these factors and what
your patient brings to the interaction will help in
dealing with frustrations related to care of this
population
QUESTIONS???
References:
1. Levenson, J. Psychiatric issues in adults with sickle cell disease. Primary Psychiatry 2008; 15(5):45-49.
2. Bandeira, I., Rocha, L., Barbosa, M., et al. Chronic inflammatory state in sickle cell anemia patients is
associated with HBB*S haplotype. Cytokine. 2014; 65(2): 217-221.
3. Platt, A., Eckman, J., Beasley, J., et al. Treating sickle cell pain: an update from the Georgia
comprehensive sickle cell center. J Emerg Nurs 2002; 28:297-303.
4. Lutz, B., Meiler, S., Bekker, A., et al. Updated mechanisms of sickle cell disease-associated chronic pain.
Transl Perioper Pain Med. 2015; 2(2):8-17.
5. McClish, D., Penberthy, L, Bovbjerg, V, et al. Health related quality of life in sickle cell patients: the
PiSCES project. Health and Qual Life Outcomes. 2005; 3(1): 1-7.
6. Levenson, J., McClish, D., Dahman, B., et al. Depression and anxiety in adults with sickle cell disease: the
PiSCES project. Psychosom Med. 2008; 70(2): 192-196.
7. Slavich, G. and Irwin, R. From stress to inflammation and major depressive disorder: a social signal
transduction theory of depression. Psychol Pull. 2014; 140(3): 774-815.
8. Miller, A., Maletic, V., and Raison, C. Inflammation and its discontents: the role of cytokines in the
pathophysiology of major depression. Biol Psychiatry. 2009; 65(9): 732-741.
9. Singhal, G., Jaehne, E., Corrigan, F., et al. Inflammasomes in neuroinflammation and changes in brain
function: a focused review. Front Neurosci. 2014; 8(315): 1-13.
10. Wills, K., Nelson, S., Hennessy, J., et al. Transition planning for youth with sickle cell disease embedding
neuropsychological assessment into comprehensive care. Pediatrics. 2010; 126 (suppl 3): S151-S159.
11. Edwards, C., Mischca, T., loughlin, G., et al. A brief review of the pathophysiology, associated
pain, and psychosocial issues in sickle cell disease. Int J Behav Med. 2005; 12(3): 171-179.
References:
12. Edwards, C., O’Garo, K., Killough, A., et al. Emotional reactions to pain predict psychological distress in adult patients
with sickle cell disease. Int’l J Psychiatry Medicine. 2014; 47(1): 1-16.
13. Beck, Judith. Cognitive Therapy. John Wiley and Sons, Inc., 1979.
14. Chen, E., Coles, S., and Koto, P. A review of empirically supported psychosocial interventions for pain and adherence
outcomes in sickle cell disease. J Pediatr Psychol. 2004; 29(3): 197-209.
15. Dames-Fincher, G. A patient perspective on sickle cell disease treatment. JNMA. 1992; 84(9): 739.
16. Treadwell, M., Telfair, J., Gibson, R. W., Johnson, S. and Osunkwo, I. Transition from pediatric to adult care in sickle
cell disease: Establishing evidence-based practice and directions for research. Am. J. Hematol. 2011;86: 116–120.
17. Telfair, J., Myers, J., and Drezner, S. Transfer as a component of the transition of adolescents with sickle cell disease
to adult care: adolescent, adult and parent perspectives. Journal of Adolescent Health. 1994; 15(11): 558-565.
18. Feliu, M., Wellington, C., Crawford, R., et al. Opioid management and dependency among adult patients with sickle
cell disease. Hemoglobin. 2011; 35(5-6): 485-494.
19. Elander, J., Lusher, J., Bevan, D., et al. Understanding the causes of problematic pain management in sickle cell
disease: evidence that pseudoaddiction plays a more important role than genuine analgesic dependence. J Pain
Symptom Manage. 2004; 27(2): 156-169.
20. Freiermuth, C., Haywood, C., Silva. S., et al. Attitudes toward patients with sickle cell disease in a multicenter sample
of emergency department providers. Adv Emerg Nurs J. 2014; 36(4): 335-347.
21. Glassberg, J., Tanabe, P., Richardson, L., et al. Among emergency physicians, use of the term “sickler” is associated
with negative attitudes towards people with sickle cell disease. Am J Hematol. 2013; 86(6): 532-533.
22. Chen, E., Cole, Steve, and Kato, Pamela. A review of empirically supported psychosocial interventions for pain and
adherence outcomes in sickle cell disease. J Ped Psychol. 2004; 29(3): 197-209.